Forgot to say: that seeing other EDSers with the same symptoms and same MRIs proves that we have the EC hydrocephalus. Maybe soon doctors will stop missing it! Think of how many lives would made better!
Yes, here’s hoping one or more such doctors reads this. There’s such a shortage of knowledge out there (and a gross shortage of experienced Neurologists), so doctors are left with a bit of a dilema, they have a patient to treat with many symptoms and not a clue what the root of the cause is, or how to help them.
Here’s my take on it. At some point there will have been an ‘initiating event’, some like myself describe head trauma (sometimes long before symptoms manifest), some like Dr Diana describe a virus as the triggering event but whatever it was, it will have made the brain swell. Now as the skull is a rigid container, anything swelling inside will have to find space to swell into, which is usually the hole at the bottom of the skull, where the spinal cord comes through. So the bottom of the cerebellum (the hind-brain) herniates and starts to peep out of this opening. Normally this space is occupied by Cerebro Spinal Fluid (CSF), passing freely between the head and the neck.
Once the hind brain takes up this space, it starts acting as a ‘bung’ and blocks or restricts the free flow of CSF. This ‘blocking’ of normal CSF flow, additionally leads to a build-up of CSF around brain, sometimes referred to as Cerebral Edema, or Hydrocephalus, which raises intra-cranial pressure. When the pressure becomes raised in the intra-cranial space, all sorts of problems are caused.
It’s now harder for the heart to get oxygenated blood into the brain, so it either has to beat faster (causing tachycardia) or harder (causing high blood pressure), both are undesirable for any length of time. If these two mechanisms fail, the brain starts to malfunction further.
A secondary problem is that, over time, the raised intracranial pressure causes the CSF to invade the Sella (or Sella Turcica). The Sella Turcica is a special little bony chamber, in the middle of the base of the brain, which is there to protect the Pituitary Gland. This is a very important, cherry shaped gland, which is the master control for the endocrine system, which deals with all kinds of essential hormones. In it’s early stage this invasion is referred to as ‘Partially Empty Sella’ as the pituitary gland becomes compressed but usually still works - however, it is a warning of things to come! Once the pituitary gland is flattened, it’s often malfunctioning and probably will have been for a while. At this stage it is referred to, on MRI’s, as an ‘Empty Sella’, which is also shown on the MRI example above. Few radiologists, or neurologists for that matter, appreciate the significance of this ‘medical sign’.
The raised ICP pressure can compress the Hypothalamus and Brain-stem too and contribute towards messing up the autonomic nervous system, which is the master control for major systems and the organs of the body - no wonder ours don’t work properly!!! Dysfunctional heartrate, blood-pressure, circulation, temperature, respiration and digestion all play real havoc with the body.
All this in itself is bad enough but, if you are then unfortunate enough to suffer a whiplash injury, that results in damage to the ligaments which hold the head on (at the cranio-cervical junction) - er, that’ll be me, then it’s a whole new ball game. Suddenly the head want’s to slide backwards and forwards (albeit only slight) but, if the piece of anatomy that’s in it’s path is that rogue piece of hind-brain, you’re heading for trouble, because directly in front of this is your BRAIN-STEM, get the picture ? Worsening autonomic dysfunction, messages interrupted between brain and body. Many, many intermittent symptoms, impossible to test for when not symptomatic and often earning the poor sufferer the label of ‘nutcase’.
I think people with the hypermobility type of EDS, whose ligaments are s-t-r-e-t-c-h-y may have an underlying, craniocervical instability too, which is allowing their head to subtly compress both their hind-brain and their brain-stem, as above.
Maybe what these people need are:-
1) Something to stabilize the head (e.g. a Philadelphia Collar).
2) Something to reduce the ICP (e.g Diamox or Acetazolamide).
3) Something to help improve their Collagen production (Vit C, protein).
4) Maybe something to help reduce any brain swelling (Antihistamine).
5) Drugs necessary to support organs and systems that are not functioning properly:
Maybe Growth Hormone.
Maybe Thyroid Hormone.
6) Supplements of essential minerals (e.g Magnesium, Zinc, etc) and vitamins (e.g. multi B Vitamins, also Vitamin D) that have depleted through not being able to look after
themselves properly, from being disabled indoors, or simply from having a digestive system that doesn’t work properly.
7) Muscle massage to help with discomfort and improve functionality.
8) Lymphatic drainage to aid a sluggish system and help speed up the process of getting rid of built-up toxins.
9) Someone to help at home, to lessen the burden of pain and stress, whilst healing takes place.
10) A diet including Magnesium rich foods, wherever possible.